To investigate the clinical features of secondary hemophagocytic syndrome (HPS), or named haemophilic lymphohistiocytic hyperplasia (HLH).

A case of secondary HPS with liver failure as the main manifestation from Department of Infections Disease of Shiyan People′s Hospital on September 27, 2017 was introduced, and literatures were reviewed.

The clinical manifestations of the patient were fever and splenomegaly, blood tests showed that hemoglobin was 88 g/L, platelet was 36×10⁹/L, neutrophil count was 0.72×10⁹/L, triglyceridemia was 3.8 mmol/L, fibrinogen was 1.15 g/L, natural killer percentage was 0.02%, ferritin was 2 000.0 μg/L, and lactate dehydrogenase was 560 U/L. According to the above test results, HPS could be diagnosed. In line with the diagnostic criteria for acute liver failure, the level of serum total bilirubin was 189.6 μmol/L, the prothrombin activity was 39.1%, the level of serum alanine aminotransferase was 279 U/L, the level of serum aspartic aminotransferase was 221 U/L.

Liver failure can be the main manifestation of secondary HPS, and scientific diagnosis of secondary HPS is crucial.